This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. This research is studying the effects of Arikace, in people with cystic fibrosis [CF] who have chronic Pseudomonas aeruginosa lung infections. We will be measuring the amount of study drug in the blood, the urine, and the sputum at different times after the study drug is taken to see how the body processes the drug. We are also testing whether certain substances in the blood can help us to predict a persons response to the study drug. Arikace, an antibiotic, has not been approved by the U.S. Food and Drug Administration [FDA]. Combined results from prior studies showed improvement in lung function and a decrease in the number of bacteria in the sputum of CF patients. Arikace is a variation of the drug Amikacin, which is approved by the FDA to treat infections caused by Pseudomonas and some forms of Mycobacteria. Arikace has been made so that the antibiotic is contained inside a fatty substances called lipids. In this study, the Arikace will be inhaled using a nebulizer machine. The researchers hope that breathing in this medication will target the lung infection more effectively.